Background: Carcinoid syndrome is a rare disease caused by malignant neuroendocrine neoplasms. When vasoactive substances enter the systemic circulation, the triad of cutaneous flushing, bronchospasm, and diarrhoea often characterize carcinoid syndrome. Rarely, carcinoid syndrome can progress to involve the cardiac system, a condition known as carcinoid heart disease, often affecting right-sided valvular structures.
Case summary: Here, we present a case of malignant carcinoid syndrome with associated carcinoid heart disease in a 63-year-old female. The diagnosis of her dual regurgitant and stenotic valvular disease is detailed, with accompanying two- and three-dimensional echocardiographic images demonstrating the patient's complex tricuspid dysfunction.
Discussion: Carcinoid heart disease encompasses a rare but important subset of valvular dysfunction caused by circulating vasoactive substances. Diagnosis utilizing serum studies, computed tomography scans, and echocardiography can help expedite the diagnosis and treatment of such rare conditions, and assist in the avoidance of complications. Despite its relatively well-recognized clinical presentation, carcinoid syndrome and its associated heart disease still remains a challenging condition to manage and treat, often requiring the input of several subspecialties to treat the condition appropriately.
Keywords: Carcinoid heart disease; Case report; Neuroendocrine neoplasms; Tricuspid regurgitation.
© The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology.